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We present a case of a nine-year old patient with MIS-C who developed a second-degree atrioventricular block upon initial presentation. This report illustrates the importance of recognizing myocardial conduction abnormalities and further cardiovascular monitoring in patients with MIS-C who require prompt intensive care management.

Vaskulitisi su rijetke reumatske bolesti nepoznate etiologije kojima je osnovno obilježje nekrotizirajuća upala krvnih žila. Posebnu skupinu čine granulomatozni vaskulitisi, izrazito rijetko opisivani u dječjoj dobi. Prikazujemo dvije bolesnice s Takayasuovim arteritisom (TA) kao entitetske oblike rijetkih reumatskih bolesti. Jedna je bolesnica imala TA tip II. a, a druga tip IV. U prve bolesnice nalazimo teške simptome opstrukcijskih lezija aortnih ogranaka, osobito tešku stenozu koronarnih arterija i okluziju lijeve potključne arterije te stenozu torakalne aorte ispod istmusa. Bolest je dijagnosticirana u akutnoj fazi, liječena je opsežno medikamentozno (glukokortikoidi, citostatici, metotreksat) i složenim kardiokirurškim pristupom, a zbog recidiva korištena je i biološka (rituksimab) terapija. Druga je bolesnica otkrivena zbog simptomatske arterijske hipertenzije, s izostankom pulseva na donjim udovima, a razlog tome nađen je u teškom suženju aorte od dijafragme do bifurkacije femoralnih arterija (mid aortic sindrom). U trenutku dijagnoze sama bolest nije bila u aktivnoj fazi. Liječena je osobitim kardiokirurškim pristupom i polimedikamentno zbog recidiva. Obje su bolesnice u adolescentnoj dobi i uspješno se liječe uz zadovoljavajuću kvalitetu života. Tip II. a s dodatnom okluzijom koronarnih krvnih žila nije prikazan u dostupnoj literaturi. Opisani vaskulitisi još uvijek snažno povezuju pedijatrijsku kardiologiju i reumatologiju, a prikaz svjedoči o važnosti timskog rada pedijatrijskih kardiologa i reumatologa., Vasculitides are rare rheumatic diseases of unknown etiology whose main characteristic is a necrotizing infl ammation of blood vessels. We are presenting two patients with Takayasu arteritis (TA) as entity forms of rare rheumatic diseases. One patient had TA type IIa and the other type IV. In the fi rst patient we found severe symptoms of obstructive lesions of aortic branches, particularly severe coronary artery stenosis and complete occlusion of the left subclavian artery, and thoracic artery stenosis below the isthmus. Th e disease was diagnosed in the acute phase, treated extensively with medicaments (glucocorticoids, cytostatics, methotrexate) and a complex cardiac surgical procedure, and due to relapse the biological (Rituximab) therapy was used. Th e second patient was detected following symptomatic arterial hypertension, with absent pulses of lower limbs, whose cause was found in severe narrowing of the aorta from diaphragm to femoral arteries bifurcation (mid-aortic syndrome). Th e disease was not active when diagnosis was made. Th e patient was treated with a particular cardiac surgical procedure and with multiple medicaments due to a relapse. Both patients have reached adolescent age and are successfully treated with a satisfying quality of life. Type IIa with an additional occlusion of coronary arteries is not described in the available literature. Forementioned vasculitides emphasize the importance of pediatric cardiologists and rheumatologists teamwork.

Although bicuspid aortic valve (BAV) is considered the most common congenital heart defect (CHD) in adult age, with the 0.5-2% prevalence, BAV is not part of epidemiological studies of congenital heart defects (CHD) in children. Aortic valvulogenesis disorder is part of the left ventricular outflow tract (LVOT) genetic disorders which include: hypoplastic left heart syndrome (HLHS), aortic stenosis (AS) and insufficiency (AI), dilatation of the ascending aorta (DAA), coaretation of the aorta (CoA), Shone's syndrome (SS), and probably some other disorders. Our observations indicate that BAV related significant pathologic and hemodynamic changes occur in children already. In an 11-year long retrospective study (2000-2011) we have found 229 BAV patients, predominantly males (1.7). The most common BAV associated disorder was CoA (75 patients, 32.6%). Of all the children with BAV, 62.4% (143:229) had hemodynamic alterations on the aortic valve which manifested themselves as aortic stenosis and/or insufficiency. AS is mostly progressive and becomes hemodynamically relevant in childhood age already, while AI is mostly mild and rarely hemodynamically relevant. A large proportion of patients had isolated AS with DAA (21 or 14.7%), while most patients had combined AS and AI (29 or 20.3%). Due to morphological changes on the valve itself and on the adjoining defects, numerous interventional and cardiosurgical procedures have been performed. Their number has been growing with age, in accordance with the expected progression of pathological changes on the valve (AS, AI) or on the aorta (DAA). DAA in children with BAV was found in 76 (33.2%) patients, in various combinations with other associated LVOT anomalies. Already in childhood have the following surgical procedures on children with primary BAV diagnosis been performed : resection of CoA with T-T anastomosis was performed in 56 patients (24.5%); balloon aortic valvuloplasty in 28 patients (12.3%); commissurotomy in 19 patients (8.3%); balloon dilatation of CoA in 15 patients (6.5%); subaortic membrane resection in 11 patients (4.8%); Ross procedure in 8 patients (3.5%); resection of CoA with reconstruction in 8 patients (3.5%); valvuloplasty in 6 patients (2.6%); ascending aortoplasty in 5 patients (2.2%); mechanical valve replacement in 3 patients (1.3%); "subclavian flap" in 3 patients (1.3%); biological aortic valve replacement in 2 patients (0.9%); Bentall procedure in 1 patient (0.4%); David procedure in 1 patient (0.4%). CONTRIBUTION OFTHE STUDY: A BAV finding in children is a predictive factor for a progressive development of morphological changes in various LVOT parts, requiring that in some patients hemodynamic repercussions be removed already in childhood.The term valvular aortopathy, that is bicuspid aortic valve syndrome, should be in use already for children, and the anomaly should be included in epidemiological CHD research.

Uvod: Aritmogena kardiomiopatija desne klijetke (engl. Arrhythmogenic right ventricular cardiomyopathy – ARVC) nasljedna je kardiomiopatija karakterizirana pretvorbom miocita u masno i vezivno tkivo, pretežno desne, no ­nerijetko i lijeve klijetke. Važan je uzrok iznenadne smrti djece i adolescenata. Temeljita obiteljska anamneza i suvremeni dijagnostički i terapijski pristup preduvjeti su za sprečavanje sindroma iznenadne smrti. Češće se dijagnoza postavlja u odraslih nego u djece. Prikaz bolesnika: Prikazujemo obitelj u kojoj je u četvero članova postavljena dijagnoza ARVC-a već u dječjoj/adolescentnoj dobi. Prosječna dob pojave simptoma kod tih bolesnika bila je 12 godina (10 – 14). Ističe se važnost obiteljskog stabla i opisuju se suvremene metode dijagnosticiranja i terapijskog pristupa, radi prevencije iznenadne smrti. Zaključujemo da je obiteljska anamneza temelj za rano postavljanje dijagnoze ARVC-a. Nazire se potreba revizije postojećih dijagnostičkih kriterija ARVC-a za pedijatrijsku populaciju. Valjalo bi uzeti u obzir progresivno povećanje ­udjela veziva u odnosu prema miocitima u ovisnosti o dobi i stadiju bolesti. S obzirom na neprimjenjivost kriterija smetnja repolarizacije kod djece mlađe od 14 godina, treba razmotriti smanjenje broja kriterija potrebnih za postavljanje dijagnoze u toj dobnoj skupini. Implantabilni elektrokardioverter defibrilator (ICD) i radiofrekventna ablacija u kombinaciji kratkoročno osiguravaju odličnu kvalitetu života kod našeg bolesnika s teškim oblikom ARVC-a, dok su dugoročni rezultati s obzirom na progresivni tijek bolesti neizvjesni., Introduction: Arrhytmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterized by fibrofatty replacement, dominantly in the right, but often also in the left ventricle. It is a significant cause of sudden death in children and adolescents. A thorough family history and contemporary diagnostic and treatment approach are prerequisites for the prevention of the sudden death syndrome. The diagnosis is more often established in adults than in children. Patients: We present a family with four members affected and diagnosed with ARVC already in childhood/adolescence. The average age of symptom presentation was 12 years (10–14 years). The importance of family tree isemphasized and contemporary diagnostic and treatment methods, with the aim of sudden death prevention, are described. Conclusions: Family history is essential for the early ARVC diagnosis. There is a need for revision of current diagnostic criteria in pediatric population. In the future progression of fibrous tissue proportion in relation to age and the stage of the disease should be taken in account. Furthermore, the repolarisation abnormality is inappropriate as a criterion in children less than 14 years old, so in that age group less of present diagnostic criteria can be applied. A combination of modern implantable cardioverter defibrillator and radiofrequency ablation assures an excellent life quality in our patients. However, the longterm prognosis for our patients, because of a progressive course of the disease, remains uncertain.

Naša je studija klinička epidemiološka retrospektivna analiza koarktacije aorte u vremenskom slijedu od 10 godina (2001. – 2011.). U studiju je uključeno 201 dijete, 72 (35,82%) djevojčice i 129 (64,18%) dječaka (1 : 1,6) prosječne dobi kod postavljanja dijagnoze 28,57 ± 49,37 mjeseci (0,1 – 204). Razvrstani su u 4 dobne skupine: novorođenačka dob (< 1 mjeseca), 1 mjesec – 1 godine, 1 – 6 godina i > 6 godina. Izoliranu koarktaciju imalo je 125 (62,19%) bolesnika, od čega 33 (16,42%) preduktalnu i 92 (45,77%) postduktalnu, a njih 76 (37,81%) imalo je koarktaciju s dodatnom srčanom grješkom; s ventrikularnim septalnim defektom 32 (15,92%), u okviru kompleksne srčane grješke 28 (13,93%), u sklopu Shoneova kompleksa 11 (5,47%) i s dilatacijskom kardiomiopatijom 5 (2,49%). U mlađim dobnim skupinama (novorođenačka i dojenačka dob) prevladava zamaranje, pojačano znojenje i dispnoičke tegobe, a kod starije djece klaudikacije, glavobolja i epistaksa. Kod čak 61 (30,35%) ispitanika dogodio se previd dijagnoze na prethodnome kardiološkom pregledu. Kod 20 (9,95%) bolesnika koarktacija se nalazi u okviru poznatih sindromnih bolesti (Turner, Noonan, Williams-Beuren, Ellis-van Creveld, parcijalna trisomija 18, Downov sindrom, fetalni valproatni sindrom). Ehokardiografija je učinjena u svih bolesnika, a kod 45 (22,38%) bila je i jedina slikovna dijagnostička pretraga. Učinjene su ukupno 123 kateterizacije srca, 38 kompjutoriziranih tomografija i 15 magnetskih rezonancija. Gradijent na mjestu koarktacije mjeren prije operacije ili interventnog liječenja izmjeren na kateterizaciji srca kod 132 (65,67%) bolesnika iznosio je 57,99 ± 18,68 mmHg (20 – 100 mmHg). Kod 82 (40,80%) bolesnika nađena je bikuspidalna aortalna valvula. Prosječna dob kod operacije bila je 27,92 ± 47,98 mjeseci (0,1 – 204). Kod 169 (84,07%) izvršena je kardiokirurška korekcija, od toga T-T-anastomoza kod 109 (54,23%), a kod 30 (14,92%) učinjena je terapijska kateterizacija (balonska dilatacija ili implantacija stenta). Smrtni ishod zabilježen je u četvero djece (1,99%), svi u novorođenačkoj ili dojenačkoj dobi., Our study is a clinical epidemiological retrospective analysis of coarctation of the aorta in a 10-year follow-up (2001–2011). The study includes 201 children, 72 (35.82%) girls and 129 (64.18%) boys (1:1,6), with an average age of 28.57±49.37 mo (0.1 – 204 mo). They are categorized in 4 age groups: 6 years. Isolated coarctation of the aorta was found in 125 (62.19%) patients; 33 (16.42%) preductal and 92 (45.77%) postductal. Coarctation of the aorta with an additional heart defect was found in 76 (37.81%) patients; 32 (15.32%) with ventricular septal defect, 28 (13.93%) within a complex heart defect, 11 (5.47%) within the Shone syndrome, and 5 (2.49%) with a dilated cardiomyopathy. Tiredness, intense tachypneic and dyspneic difficulties are dominant features in lower age groups (newborns and infants), while claudications, headaches and epistaxis are typical in older children. In the case of as many as 61 (30.35%) patients diagnosis was missed on the previous cardiological examination. In 20 (9.95%) patients coarctation is found within the known syndromes (Turner, Noonan, Williams Beuren, Ellis van Creveld, Down, partial trisomy 18, fetal valproate syndrome). Echocardiography was performed in all patients, and in 45 (22.38%) it was the only diagnostic procedure. Altogether, 123 heart catheterizations, 38 multislice computed tomography and 15 magnetic resonance imagings were performed. The gradient on the place of coarctation before surgery or emergency procedures measured by catheter in 132 (65.67%) patients was 57.99±18.68 mmHg (20–100 mmHg). In 82 (40.80%) patients a bicuspid aortic valve was found. Average age at the time of surgery was 27.92±47.98 months (0.1–204 mo.). In 169 (84.07%) patients a cardiosurgical intervention was performed; 109 (54.23%) T-T anastomoses, 30 (14.29%) therapeutic catheterisations (balloon dilatation or stent implantation). Fatal outcome occurred in 4 (1.99%) children, all newborns or infants.

Bikuspidalna aortalna valvula (BAV) epidemiološki se ne uvrštava u studije prirođenih srčanih grješaka (PSG) u djece iako se u odrasloj dobi smatra najčešćim PSG-om, s prevalencijom od 0,5 do 2%. Poremećaj aortalne valvulogeneze posljedica je genski uvjetovanih promjena u izlaznom dijelu lijeve klijetke (LVOT) u koje spadaju: sindrom hipoplastičnoga lijevog srca (HLHS), aortalna stenoza (AS) i insuficijencija (AI), dilatacija uzlazne aorte (DAA), koarktacija aorte (CoA), Shoneov sindrom (SS), a vjerojatno i neki drugi poremećaji. Mi smo zapazili da su uz BAV vezane znatne patomorfološke promjene već u dječjoj dobi. U retrospektivnu studiju dugu 11 godina (od 2002. do 2012.) uključeno je 229-ero djece s BAV-om, s predominacijom muških (1,7). Najčešća grješka pridružena BAV-u bila je CoA (75 bolesnika ili 32,6%). Od djece s BAV-om njih 62,4% (143 : 229) imalo je hemodinamske promjene na aortalnoj valvuli koje se očituju kao aortalna stenoza i/ili insuficijencija. AS je većinom progresivan i postaje već u dječjoj dobi hemodinamski važan u dijela bolesnika, a AI je većinom blag i rijetko hemodinamski važan.Velik broj bolesnika imao je izolirani AS uz DAA (21 ili 14,7%), a najveći broj imao je kombinaciju AS-a i AI (29 ili 20,3%). Zbog morfoloških promjena na samoj valvuli i pridruženim grješkama učinjeni su brojni intervencijski i kardiokirurški zahvati. Njihov broj rastao je s dobi, sukladno očekivanoj progresiji patoloških promjena na samoj valvuli (AS, AI) ili na aorti (DAA). Kod djece s BAV-om nalazimo DAA u 76 (33,2%) bolesnika, i to u raznim kombinacijama s drugim grješkama LVOT-a. Uz osnovnu dijagnozu BAV-a već u dječjoj dobi učinjene su ove operacije: resekcija CoA s T-T-anastomozom 56 bolesnika (24,5%), balonska dilatacija BAV-a 28 bolesnika (12,3%), komisurotomija 19 bolesnika (8,3%), balonska dilatacija CoA 15 bolesnika (6,5%), resekcija subaortalne membrane 11 bolesnika (4,8%), operacija prema Rossu 8 bolesnika (3,5%), resekcija CoA s rekonstrukcijom 8 bolesnika (3,5%), valvuloplastika 6 bolesnika (2,6%), plastika ascendentne aorte 5 bolesnika (2,2%), mehanička aortalna valvula 3 bolesnika (1,3%), potključni režanj (subclavian flap) 3 bolesnika (1,3%), biološka aortalna valvula 2 bolesnika (0,9%), operacija prema Bentallu 1 bolesnik (0,4%), operacija prema Davidu 1 bolesnik (0,4%). Doprinos studije: Nalaz BAV-a kod djece prediktivni je čimbenik (navješćivač) progresivnog razvoja morfoloških promjena u različitim dijelovima LVOT-a, s potrebom za uklanjanjem hemodinamskih reperkusija u dijela bolesnika već u dječjoj dobi. Zaključak: Pojam valvularna aortopatija, odnosno sindrom bikuspidalne aortalne valvule trebalo bi rabiti već u dječjoj dobi, a anomaliju valja uključiti u epidemiološka istraživanja PSG-a., Although bicuspid aortic valve (BAV) is considered the most common congenital heart defect (CHD) in adult age, with the 0.5-2% prevalence, BAV is not part of epidemiological studies of congenital heart defects (CHD) in children. Aortic valvulogenesis disorder is part of the left ventricular outflow tract (LVOT) genetic disorders which include: hypoplastic left heart syndrome (HLHS), aortic stenosis (AS) and insufficiency (AI), dilatation of the ascending aorta (DAA), coarctation of the aorta (CoA), Shone’s syndrome (SS), and probably some other disorders. Our observations indicate that BAV related significant pathologic and hemodynamic changes occur in children already. In an 11-year long retrospective study (2000-2011) we have found 229 BAV patients, predominantly males (1.7). The most common BAV associated disorder was CoA (75 patients, 32.6%). Of all the children with BAV, 62.4% (143:229) had hemodynamic alterations on the aortic valve which manifested themselves as aortic stenosis and/or insufficiency. AS is mostly progressive and becomes hemodynamically relevant in childhood age already, while AI is mostly mild and rarely hemodynamically relevant. A large proportion of patients had isolated AS with DAA (21 or 14.7%), while most patients had combined AS and AI (29 or 20.3%). Due to morphological changes on the valve itself and on the adjoining defects, numerous interventional and cardiosurgical procedures have been performed. Their number has been growing with age, in accordance with the expected progression of pathological changes on the valve (AS, AI) or on the aorta (DAA). DAA in children with BAV was found in 76 (33.2%) patients, in various combinations with other associated LVOT anomalies. Already in childhood have the following surgical procedures on children with primary BAV diagnosis been performed : resection of CoA with T-T anastomosis was performed in 56 patients (24.5%); balloon aortic valvuloplasty in 28 patients (12.3%); commissurotomy in 19 patients (8.3%); balloon dilatation of CoA in 15 patients (6.5%); subaortic membrane resection in 11 patients (4.8%); Ross procedure in 8 patients (3.5%); resection of CoA with reconstruction in 8 patients (3.5%); valvuloplasty in 6 patients (2.6%); ascending aortoplasty in 5 patients (2.2%); mechanical valve replacement in 3 patients (1.3%); »subclavian flap« in 3 patients (1.3%); biological aortic valve replacement in 2 patients (0.9%); Bentall procedure in 1 patient (0.4%); David procedure in 1 patient (0.4%). Contribution of the study: A BAV finding in children is a predictive factor for a progressive development of morphological changes in various LVOT parts, requiring that in some patients hemodynamic repercussions be removed already in childhood. Conclusion: The term valvular aortopathy, that is bicuspid aortic valve syndrome, should be in use already for children, and the anomaly should be included in epidemiological CHD research.

2016;11(3-4):135. VII. nacionalni sastanak o kardiovaskularnim intervencijama s međunarodnim sudjelovanjem VI. sastanak intervencijskih kardioloskih medicinskih sestara i tehnicara Development of interventional cardiology in treating heart diseases in children in the Reference Centre for Pediatric Cardiology1 in the last 20 years (1996 2015) is presented. Constant growth in the number of heart catheterizations (from 160 to 241) is followed by a relatively higher growth in the number of interventional catheterizations (from 12.5% to 38.1%). While the number of catheterizations (Table 1) has increased by 50% (160 to 241 =1,5), the number of interventional catheterizations has increased by more than 200% (12.5 – 38.1% > 3). In our conditions these data point to an absolute increase of interventional treatment when compared to the diagnostic heart catheterization itself, despite the fact that the latest diagnostic methods are still not applied sufficiently (MSCT, MRI, Ivan Malcic*, Dalibor Saric, Hrvoje Kniewald, Daniel Dilber, Dorotea Bartonicek, Darko Anic, Visnja Ivancan, Dražen Belina, Alen Hodalin

We conducted a retrospective study at the Department of Paediatric Cardiology of the University Hospital Centre Rebro, Zagreb, over the period from 1988 to 1998, so as to assess the epidemiology of childhood cardiomyopathies. The patients were categorized according to the guidelines of the Task Force on Cardiomyopathies of the World Health Organization and the International Society and Federation of Cardiology. We identified 121 infants, children and adolescents as having cardiomyopathy, giving an average occurrence for all cardiomyopathies of 38.81 for each 10, 000 patients examined in our outpatient clinics for paediatric cardiology. Of the patients, 50 were female (41.3%) and 71 were male (58.7%). The cardiomyopathy was of the dilated variant in 52 patients (42.9%), with 43 patients (35.5%) having hypertrophic cardiomyopathy, and 6 patients (4.8%) identified with restrictive cardiomyopathy. We encountered no patients with arrhythmogenic right ventricular cardiomyopathy. In nine patients (7.4%), it proved impossible to classify the cardiomyopathy. We placed 11 patients (9.0%) in the group of specific cardiomyopathies. Most of those with dilated cardiomyopathy had been diagnosed prior to the age of 3 years (RR 1.9, 95% CI 1.4-2.47). There were no statistically significant differences in the incidences of dilated as compared to hypertrophic cardiomyopathy (Z 0.923, p = 0.1779), but we encountered a significantly lower occurrence of restrictive cardiomyopathy (Z 6.044, p < 0.001). Of those with hypertrophic cardiomyopathy, 15 patients (34.8%) had the asymmetric variant, while 28 patients (65.2%) exhibited the concentric form. During the period of follow-up, 10 patients died, 4 with dilated cardiomyopathy, 4 with hypertrophic cardiomyopathy, 1 with restrictive cardiomyopathy, and 1 with a specific cardiomyopathy. We encountered 12 (9.9%) patients who, besides cardiomyopathies, also suffered from neuromuscular disorders. Most of these had dilated cardiomyopathy. Mitochondrial disorders, in contrast, were more frequently found in patients with hypertrophic cardiomyopathy.

Koronarna je arterijska fistula komunikacija između jedne ili više koronarnih arterija s nekom drugom šupljinom u srcu ili segmentom sistemskog ili pulmonalnog optoka. Arterijska krv iz koronarne krvne žile ulazi u drugi segment preko miokardijalnoga kapilarnog bazena. To su vrlo rijetke anomalije, a učestalost im se procjenjuje na 0,2 – 0,4% svih prirođenih srčanih grješaka. Ipak, one su klinički važne ako su srednje velike ili velike te se očituju nizom kliničkih simptoma kao što su angina pectoris, aritmije, miokardijalni infarkt, endokarditis, progresivna dilatacija, srčana insuficijencija s kardiomiopatijom, plućna hipertenzija, tromboza fistula i razvoj aneurizme s mogućom rupturom. Ovdje prikazujemo šest bolesnika s koronarnom arterijskom fistulom uz opis toka bolesti, dijagnostičkih postupaka i ishoda bolesti. Terapijsko zatvaranje koronarnih fistula preporučuje se kod svih simptomatskih, ali i kod asimptomatskih bolesnika, i to ako postoje značajni laboratorijski znakovi bolesti, u prvom redu rendgenske ili elektrokardiografske abnormalnosti. Transkatetersko zatvaranje koronarnih fistula u novije je vrijeme moguća zamjena kardiokirurškom pristupu i sve se više rabi zahvaljujući većim dijagnostičkim mogućnostima i sve boljim tehnološkim rješenjima. Danas se kod pedijatrijskih bolesnika daje prednost intervencijskom zatvaranju fistula ako je to moguće. Odabir metode ovisi o anatomiji fistule, prisutnosti ili odsutnosti dodatnih defekata te o iskustvu intervencijskog kardiologa ili kardijalnog kirurga. Učinak obiju metoda je dobar ako se uspješno izvedu. U našem je radu prikazano dvoje djece s fistulom između desne koronarne arterije i desne klijetke (RV), jedno dijete s fistulom između LAD-a i RV-a, jedno dijete s fistulom između glavnog stabla lijeve koronarne arterije (LCA) i RV-a, jedno dijete s fistulom između LCA i izlaznog trakta desne klijetke (RVOT-a) i jedno dijete s fistulom između LCA i desne pretklijetke (RA). Ova posljednja (LCA – RA) fistula nije opisana u najnovijoj klasifikaciji anomalija koronarnih krvnih žila kod djece na osnovi MSCT koronarografije pa svoj prikaz smatramo doprinosom novoj klasifikaciji. Uz opise različitih fistula i prikaze intervencijske i kardiokirurške intervencije prikazujemo i rijedak slučaj spontanog zatvaranja tijekom prvih šest mjeseci i ponovnog otvaranja fistule nakon 6 godina između desne koronarne arterije i desne klijetke., A coronary artery fistula is a link between one or more coronary arteries with another heart cavity or a segment of systemic or pulmonary circulation. Arterial blood from a coronary vessel enters another segment via myocardial capillary bed. These are very rare anomalies which constitute approximately 0.2 – 0.4% of all congenital heart defects. Still, they are clinically significant if they are of medium or large size and are manifested with a series of clinical symptoms such as angina pectoris, arrhythmias, myocardial infarction, endocarditis, progressive dilatation, heart failure and cardiomyopathy, pulmonary hypertension, thrombosis of the fistula and formation of aneurysms with possible ruptures. We present six patients with a coronary arterial fistula, their history, diagnostic procedures and outcomes. Therapeutic closure of coronary artery fistulas is recommended in all symptomatic, but also in asymptomatic patients, if there are significant roentgenographic, electrocardiographic and other abnormalities. In recent times transcatheter closure of coronary fistulas has become a possible alternative to surgery and is becoming increasingly used thanks to improved diagnostic possibilities and technology. If possible, interventional closure of fistulas is precisely the method preferred in pediatric patients. The choice of method depends on the anatomy of the fistula, presence or absence of additional defects, and on the experience of an interventional cardiologist or a heart surgeon. If performed well, the effects of both methods are good. This paper presents two children with a fistula between the right coronary artery and the right ventricle (RV), one child with a fistula between LAD and RV, one child with a fistula between the main tree of the left coronary artery (LCA) and RV, one child with a fistula between LCA and the right ventricular outflow tract (RVOT), and one child with a fistula between LCA and the right atrium (RA). The last one (LCA-RA) is not described in the latest classification of anomalies of coronary blood vessels in children based on MSCT coronarography, so we consider our presentation to be a contribution to the new classification. Along with the descriptions of fistulas and presentations of interventional and cardiosurgical interventions, we are also presenting a rare case of spontaneous closing of the fistula within the first six months and of a reopening of the fistula between the right coronary artery and the right ventricle after six years.

Background: The children of male veterans with combat-related posttraumatic stress disorder (PTSD) are at particularly high risk of emotional and behavioral problems. However, no studies have examined non-suicidal self-injury (NSSI) in this population of youth. Aims: To determine the prevalence and psychosocial correlates of lifetime NSSI in a sample of psychiatric outpatient adolescent offspring of Croatian PTSD male veterans. Method: Consecutive outpatient adolescent offspring of Croatian male PTSD veterans, aged 12 to 18 years, were assessed on the Deliberate Self Harm Inventory, the Youth Self-Report, the Family Assessment Device, the Parental Bonding Instrument and the Demographics Questionnaire. Results: Of the whole sample, 52.7% of adolescents reported NSSI at least once during their lifetime. Lifetime NSSI was significantly associated with internalizing symptoms (adjusted odds ratio (OR) = 2.14; 95% confidence interval (CI): 1.04–4.42, p = .040), poor family functioning (adjusted OR = 6.54; 95% CI: 2.02–21.22, p = .002), lower maternal and paternal care (adjusted OR = 0.47; 95% CI: 0.40–0.56, p = .000 and adjusted OR = 0.82; 95% CI: 0.73–0.91, p = .000, respectively) and higher paternal control (adjusted OR = 1.84; 95% CI: 1.59–2.14, p = .000) in multivariate analysis. No association was found between lifetime NSSI and any of the socio-demographic variables. Conclusion: NSSI is a significant clinical problem in outpatient adolescent offspring of PTSD male veterans, which may be influenced by clinical and family factors. Interventions aimed at reducing internalizing symptoms and improving family functioning and parental behaviors are needed in the treatment of adolescent offspring of male PTSD veterans engaging in NSSI.

All rescuers trained or not, should provide chest compressions to victims of cardiac arrest. The aim should be to push to a depth of at least 5 cm at a rate of at least 100 compressions per minute, to allow full chest recoil, and to minimise interruptions in chest compressions. Trained rescuers should also provide ventilations with a compression-ventilation ratio of 30:2. ELECTRICAL THERAPIES: Much greater emphasis on minimising the duration of the pre-shock and post-shock pauses; the continuation of compressions during charging of the defibrillator is recommended. Further development of AED programmes is encouraged. ADULT ADVANCED LIFE SUPPORT: Increased emphasis on high-quality chest compressions throughout any ALS intervention paused briefly only to enable specific interventions. Removal of the recommendation for a pre-specified period of cardiopulmonary resuscitation before out-of-hospital defibrillation following cardiac arrest unwitnessed by the EMS. The role of precordial thump is de-emphasized. Delivery of drugs via a tracheal tube is no longer recommended, drugs should be given by the intraosseous (IO) route. Atropine is no longer recommended for routine use in asystole or pulseless electrical activity. Reduced emphasis on early tracheal intubation unless achieved by highly skilled individuals with minimal interruptions in chest compressions. Increased emphasis on the use of capnography. Recognition of potential harm caused by hyperoxaemia. Revision of the recommendation of glucose control. Use of therapeutic hypothermia to include comatose survivors of cardiac arrest associated initially with shockable rhythms, as well as non-shockable rhythms, with a lower level of evidence acknowledged for the latter. INITIAL MANAGEMENT OF ACUTE CORONARY SYNDROMES: The term non-ST-elevation myocardial infarction-acute coronary syndrome (non-STEMI-ACS) has been introduced for both NSTEMI and unstable angina pectoris. Primary PCI (PPCI) is the preferred reperfusion strategy provided it is performed in a timely manner by an experienced team. Non-steroidal anti-inflammatory drugs should be avoided, as well as routine use of intravenous beta-blockers; oxygen is to be given only to those patients with hypoxaemia, breathlessness or pulmonary congestion. PAEDIATRIC LIFE SUPPORT: The decision to begin resuscitation must be taken in less than 10 seconds. Lay rescuers should be taught to use a ratio of 30 compressions to 2 ventilations, rescuers with a duty to respond should learn and use a 15:2 ratio; however, they can use the 30:2 compression-ventilation ratio if they are alone. Ventilation remains a very important component of resuscitation in asphyxial arrest. The emphasis is on achieving quality compressions with the rate of at least 100 but not greater than 120 per minute, with minimal interruptions. AEDs are safe and successful when used in children older than 1 year. A single shock strategy using a non-escalating dose of 4 J/kg is recommended for defibrillation in children. Cuffed tubes can be used safely in infants and young children. Monitoring exhaled carbon dioxide (CO2), ideally by capnography, is recommended during resuscitation. RESUSCITATION OF BABIES AT BIRTH: For uncompromised babies, a delay in cord clamping of at least one minute from the complete delivery is now recommended. For term infants, air should be used fro resuscitation at birth. For preterm babies less than 32 weeks gestation blended oxygen and air should be given judiciously and its use guided by pulse oximetry. Preterm babies of less than 28 weeks gestation should be completely covered in a plastic wrap up to their necks, without drying, immediately after birth. The recommended compression: ventilation ratio remains at 3:1 for newborn resuscitation. Attempts to aspirate meconium from the nose and mouth of the unborn baby, while the head is still on the perineum, are not recommended. If adrenaline is given the n the intravenous route is recommended using a dose of 10-30 microg/kg. Newly born infants born at term or near-term with moderate to severe hypoxic-ischaemic encephalopathy should be treated with therapeutic hypothermia. PRINCIPLES OF EDUCATION IN RESUSCITATION: The aim is to ensure that learners acquire and retain skill and knowledge that will enable them to act correctly in actual cardiac arrest and improve patient outcome. Short video/computer self-instruction courses, with minimal or no instructor coaching, combined with hands-on practice can be considered as an effective alternative to instructor-led basic life support (BLS and AED) courses. Ideally all citizens should be trained in standard CPR that includes compressions and ventilations. Basic and advanced life support knowledge and skills deteriorate in as little as three to six months. CPR prompt or feedback devices improve CPR skill acquisition and retention.

Cilj našeg rada jest prikazati porast broja intervencijskih kateterizacija srca u zadnjih 14 godina u Referentnom centru za pedijatrijsku kardiologiju RH u odnosu na broj dijagnostičkih kateterizacija. Od siječnja 1996. do prosinca 2009. godine u našem je Centru invazivnoj dijagnostici podvrgnuto 2379-ero djece, 51% muške i 49% ženske, sa srednjom dobi u trenutku invazivne dijagnostike 4,1±3,8 godina (1 dan – 20,5 godina). U tom je razdoblju kod ukupno 22,27% (2379/530) djece učinjen neki interventni zahvat na srcu. Broj se interventnih kateterizacija godišnje povećavao od 20/160 (12,5%) 1996. do 60/182 (32,9%) 2007. godine. Neke su interventne metode uvedene prije 1996. godine (balonska atrioseptostomija po Rashkindu, dilatacija pulmonalne stenoze, biopsija miokarda), a ostale su uvedene kako slijedi: dilatacija kritične aortne stenoze u novorođenčeta, kao i aortne stenoze u veće djece od 1996. godine, transkatetersko zatvaranje Botallova duktusa od 1997. godine, transkatetersko zatvaranje defekta interatrijskog septuma od 2000. godine i balonska dilatacija nativne koarktacije i rekoarktacije od 2003. godine. Od 530 interventnih kateterizacija u studiranom razdoblju bilo je 140 atrioseptostomija (26,4%), 80 dilatacija pulmonalne valvule (15,1%), 36 dilatacija aortne valvule u veće djece (6,8%), 9 dilatacija kritične aortne stenoze (1,7%), 58 dilatacija koarktacije (10,9%) – od čega 32 nativne i 26 rekoarktacija, 133 (25,1%) zatvaranja Botallova duktusa (4 PFM spiralom, 68 Cookovom spiralom i 61 Amplatzerovim kišobranom). Od 2000. godine defekt interatrijskog septuma tipa ostium secundum zatvorili smo u 37 bolesnika, od čega u 14 bolesnika Cardioseal-STARflex sustavom (37,83), a u 23 bolesnika Amplatzerovim sustavom (62,16%). Biopsiju miokarda radili smo u 37 bolesnika (7%) od čega kod petorice dva puta, a kod svake smo biopsije izvadili 4–5 uzoraka endomiokarda (većinom iz lijeve klijetke). Hi2-testom utvrdili smo stalni trend porasta broja kateterizacija u odnosu na prosječan broj (p, The goal of our study was to show an increase in the number of interventional heart catheterisations in the last 14 years in the Referral Centre for Pediatric Cardiology of the Republic of Croatia. From January 1996 to December 2009 2379 children (51% male and 49% female) underwent invasive diagnostic procedures (mean age 4.1+3.8 years, range 1 day–20.5 years). In that period in 22.27% (2379/530) of the children a certain interventional procedure was performed. The number of interventional catheterisations increased from 20/160 (12.5%) in 1996 to 70/224 (31.25%) in 2009. Some interventional procedures were introduced before 1996 (Rashkind BAS, dilatation of pulmonary valve stenosis and myocardial biopsy), while other methods were introduced in the following order: dilatation of critical aortic stenosis in newborns and other ages from 1996, ductus Bottali closure from 1997, interatrial septal defect closure from 2000, native coarctation and recoarctation of the aorta from 2003. Of 530 interventional catheterisations involved in the study during the period of 14 years, there were 140 atrioseptostomias (26.4%), 80 pulmonary valve dilatations (15.1%), 58 aortic valve dilatations in older children (6.8%), 9 dilatations of critical aortic stenosis (1.7%), 58 dilatations of coarctation (10.9%) (of which 32 native and 26 recoarctations), 133 ductus Botalli closures (25.1%) (4 with PFM coils, 68 with Cook coils and 61 with Amplatzer Duct occluder). From 2000 interatrial septal defects were closed in 37 patients, 14 of which were closed with Cardioseal-STARflex system (37.84%) and 23 with Amplatzer device (62.16%). Myocardial biopsy was performed in 37 patients (7%) with 4–5 endomyocardial samples per patient (mainly from the left ventricle) so that we had 120 heart samples in total for further analysis. Hi2-test determined a constant rise in catheterisations in relation to the mean number (p

Osnovno održavanje života odraslih. – Svi spašavatelji, bilo osposobljeni ili ne, kod žrtava kardijalnog aresta moraju primijeniti vanjsku masažu srca. Cilj je pritisnuti prsni koš do dubine od najmanje 5 cm, frekvencijom od najmanje 100 kompresija u minuti, ali i dopustiti ponovo odizanje prsnog koša, te smanjiti prekide u kompresijama. Osposobljeni bi spašavatelji trebali primijeniti i ventilaciju s omjerom kompresija-ventilacija od 30:2. Liječenje strujom. – Mnogo je veći naglasak na smanjivanju trajanja stanki prije ili poslije defibrilacije; preporučuje se nastavak vanjske masaže srca tijekom punjenja defibrilatora. Potiče se daljnji razvoj programa automatskih vanjskih defibrilatora (AED). Napredno održavanje života odraslih. – Naglašena je važnost visokokvalitetnih kompresija na prsni koš tijekom provođenja ALS-a, koje se prekidaju samo kako bi se omogućili specifični postupci. Uklanja se preporuka o potrebi reanimacije tijekom određenog vremena prije defibrilacije nakon kardijalnog aresta izvan bolnice, kojemu nije svjedočilo osoblje hitne medicinske pomoći. Smanjena je uloga prekordijalnog udarca. Primjena lijekova putem endotrahealnog tubusa više se ne preporučuje, već se lijekovi moraju primijeniti intraosealnim (IO) pristupom. Atropin se više ne preporučuje za rutinsku primjenu tijekom asistolije ili električne aktivnosti bez pulsa. Smanjen je naglasak na ranu endotrahealnu intubaciju ako ju ne provodi visokostručna osoba uz najmanji mogući prekid vanjske masaže srca. Povećan je naglasak na uporabu kapnografije. Prepoznat je moguć štetan učinak hiperoksemije. Revidirana je preporuka za kontrolu glikemije. Preporučuje se primjena terapijske hipotermije kod komatoznih bolesnika nakon kardijalnog aresta povezanog s početnim ritmovima koji se defibriliraju, kao i onima koji se ne defibriliraju, za ove druge sa smanjenom razinom dokaza. Početno zbrinjavanje akutnih koronarnih sindroma. – Uveden je pojam infarkt miokarda bez ST-elevacije–akutni koronarni sindrom (NSTEMI-ACS) koji obuhvaća infarkt miokarda bez elevacije ST-spojnice i nestabilnu anginu pektoris. Primarna PCI (PPCI) najpoželjniji je reperfuzijski postupak, uz uvjet da ga obavi iskusan tim i u skladu s vremenskim okvirima. U liječenju bi trebalo izbjegavati nesteroidne protuupalne lijekove, rutinsku intravensku primjenu beta-blokatora i kisik – osim u slučaju hipoksemije, zaduhe ili zastoja na plućima. Održavanje života djece. – Odluka o započinjanju reanimacije mora se donijeti u manje od 10 sekundi. Laike treba podučavati reanimaciji s omjerom 30 kompresija naprama 2 ventilacije, a spašavatelji koji imaju dužnost odgovoriti na poziv trebaju primjenjivati omjer kompresije-ventilacije od 15:2, međutim, ako su sami, mogu primijeniti omjer od 30:2. Ventilacija i dalje ostaje vrlo važna sastavnica reanimacije asfiksijskog aresta. Naglasak je na postizanju kvalitetnih kompresija frekvencije najmanje 100, ali ne više od 120 u minuti, uz minimalne prekide. Automatski vanjski defibrilatori sigurni su i uspješni kada se primjenjuju kod djece starije od jedne godine. Za defibrilaciju kod djece preporučuje se jedna defibrilacija od 4 J/kg čija se jačina ne povećava. Sa sigurnošću se mogu rabi endotrahealni tubusi s balončićem i kod dojenčadi i kod mlađe djece. Monitoriranje izdahnutog ugljikova dioksida (CO2), najbolje kapnografijom, preporučuje se tijekom reanimacije. Reanimacija novorođenčadi na porodu. – Kod neugrožene novorođenčadi sada se preporučuje odgađanje podvezivanja pupčane vrpce od najmanje jedne minute nakon potpunog rođenja djeteta. Za donošenu novorođenčad, tijekom reanimacije pri porodu trebao bi se rabiti zrak. Za nedonoščad mlađu od 32 tjedna gestacije treba razumno primijeniti mješavinu kisika i zraka te primjena treba biti vođena pulsnim oksimetrom. Nedonoščad mlađu od 28 tjedana gestacije trebalo bi neposredno nakon rođenja potpuno do područja vrata omotati plastičnom folijom, bez sušenja. Preporučen omjer kompresija-ventilacija u reanimaciji novorođenčadi ostaje 3:1. Ne preporučuje se pokušavati aspirirati mekonij iz nosa i usta još nerođenog djeteta, dok mu je glava još na međici. Ako se daje adrenalin, preporučuje se intravenska primjena u dozi od 10 do 30 µg/kg. Kod terminske ili gotovo terminske novorođenčadi s umjerenom do teškom hipoksično-ishemijskom encefalopatijom preporučuje se terapijska hipotermija. Načela podučavanja reanimacije. – Cilj je osigurati da polaznici steknu i zadrže vještine i znanje koje će im omogućiti ispravno djelovanje tijekom stvarnoga kardijalnog aresta te poboljšati ishod bolesnika. Kratki video/kompjutorski tečajevi za samostalno učenje, s minimalnom ulogom instruktora ili bez njega, udruženi s praktičnom nastavom mogu se smatrati jednako učinkovitom alternativom tečajevima osnovnog održavanja života (BLS i AED) koje vode instruktori. U najboljem slučaju, svi bi građani trebali biti osposobljeni za standardnu KPR koja uključuje vanjsku masažu srca i ventilaciju. Znanje i vještine osnovnog i naprednog održavanja života smanjuju se u samo tri do šest mjeseci. Uređaji s glasovnim uputama tijekom reanimacije ili povratnim informacijama unaprjeđuju stjecanje i zadržavanje vještina., Basic Life Support. – All rescuers trained or not, should provide chest compressions to victims of cardiac arrest. The aim should be to push to a depth of at least 5 cm at a rate of at least 100 compressions per minute, to allow full chest recoil, and to minimise interruptions in chest compressions. Trained rescuers should also provide ventilations with a compression-ventilation ratio of 30:2. Electrical therapies. – Much greater emphasis on minimising the duration of the pre-shock and post-shock pauses; the continuation of compressions during charging of the defibrillator is recommended. Further development of AED programmes is encouraged. Adult Advanced Life Support. – Increased emphasis on high-quality chest compressions throughout any ALS intervention paused briefly only to enable specific interventions. Removal of the recommendation for a pre-specified period of cardiopulmonary resuscitation before out-of-hospital defibrillation following cardiac arrest unwitnessed by the EMS. The role of precordial thump is de-emphasized. Delivery of drugs via a tracheal tube is no longer recommended, drugs should be given by the intraosseous (IO) route. Atropine is no longer recommended for routine use in asystole or pulseless electrical activity. Reduced emphasis on early tracheal intubation unless achieved by highly skilled individuals with minimal interruptions in chest compressions. Increased emphasis on the use of capnography. Recognition of potential harm caused by hyperoxaemia. Revision of the recommendation of glucose control. Use of therapeutic hypothermia to include comatose survivors of cardiac arrest associated initially with shockable rhythms, as well as non-shockable rhythms, with a lower level of evidence acknowledged for the latter. Initial management of acute coronary syndromes. – The term non-ST-elevation myocardial infarction-acute coronary syndrome (non-STEMI-ACS) has been introduced for both NSTEMI and unstable angina pectoris. Primary PCI (PPCI) is the preferred reperfusion strategy provided it is performed in a timely manner by an experienced team. Non-steroidal anti-inflammatory drugs should be avoided, as well as routine use of intravenous beta- blockers; oxygen is to be given only to those patients with hypoxaemia, breathlessness or pulmonary congestion. Paediatric Life Support. – The decision to begin resuscitation must be taken in less than 10 seconds. Lay rescuers should be taught to use a ratio of 30 compressions to 2 ventilations, rescuers with a duty to respond should learn and use a 15:2 ratio; however, they can use the 30:2 compression-ventilation ratio if they are alone. Ventilation remains a very important component of resuscitation in asphyxial arrest. The emphasis is on achieving quality compressions with the rate of at least 100 but not greater than 120 per minute, with minimal interruptions. AEDs are safe and successful when used in children older than 1 year. A single shock strategy using a non-escalating dose of 4 J/kg is recommended for defibrillation in children. Cuffed tubes can be used safely in infants and young children. Monitoring exhaled carbon dioxide (CO2), ideally by capnography, is recommended during resuscitation. Resuscitation of babies at birth. – For uncompromised babies, a delay in cord clamping of at least one minute from the complete delivery is now recommended. For term infants, air should be used fro resuscitation at birth. For preterm babies less than 32 weeks gestation blended oxygen and air should be given judiciously and its use guided by pulse oximetry. Preterm babies of less than 28 weeks gestation should be completely covered in a plastic wrap up to their necks, without drying, immediately after birth. The recommended compression: ventilation ratio remains at 3:1 for newborn resuscitation. Attempts to aspirate meconium from the nose and mouth of the unborn baby, while the head is still on the perineum, are not recommended. If adrenaline is given the n the intravenous route is recommended using a dose of 10–30 µg /kg. Newly born infants born at term or near-term with moderate to severe hypoxic-ischaemic encephalopathy should be treated with therapeutic hypothermia. Principles of education in resuscitation. – The aim is to ensure that learners acquire and retain skill and knowledge that will enable them to act correctly in actual cardiac arrest and improve patient outcome. Short video/computer self-instruction courses, with minimal or no instructor coaching, combined with hands-on practice can be considered as an effective alternative to instructor-led basic life support (BLS and AED) courses. Ideally all citizens should be trained in standard CPR that includes compressions and ventilations. Basic and advanced life support knowledge and skills deteriorate in as little as three to six months. CPR prompt or feedback devices improve CPR skill acquisition and retention.

Purpose of the study: (1) Analysis of competency (resuscitation knowledge and skills) of the candidates in relation to their previous/basic education. (2) Analysis of personal evaluation of the quality of the course in relation to candidates’ competency after the course. (3) Analysis of internal motivation as the most important factor that influences successful acquisition of knowledge and skills at the organised programme of continuous medical education. Material and methods: Between April 2002 and December 2009, 74 European Resuscitation Council (ERC) provider courses (55 Advanced Life Support-ALS and 19 European Paediatric Life Support-EPLS) have taken place in Croatia. Total of 1730 candidates (1328 ALS, 402 EPLS) attended. A questionnaire has been distributed to all attendees by email, handed personally or has been available on the official website of the Croatian Resuscitation Council (CroRC). Subjects covered by the questionnaire were professional background, motivation, knowledge, satisfaction with courses, applicability to everyday practice and changes in attitudes. Results: Preliminary results from 500 questionnaires collected to date show satisfactory competency of candidates in relation to their professional background, ranging from specialists and residents to nurses. Majority of candidates rated these courses with highest grades and would recommend them to other medical professionals. Higher internal motivation and satisfaction of candidates correlated well with course results. Conclusions: Based on the preliminary results from this survey, organized programme of continuous medical education in the field of reanimatology is recognised as a successful educational programme applicable to Croatian medical professionals.

U 2009. godini je u Hrvatskoj umrlo 7837 stanovnika više, nego se rodilo, stopa nataliteta je bila 10,1‰, mortaliteta 11,8‰, negativni priraštaj iznosi –1,7‰. Rođeno je 45 056 djece 500 grama (747 više nego 2008. godine), među njima 44853 djeteta 1000 grama. Učestalost nedonoščadi je 2009. godine bila 5,44%, a novorođenčadi niske porodne težine 5,55%. Perinatalni mortalitet za svu djecu 1000 g je bio 4,4‰, a za svu djecu 500 g 7,2‰. Fetalni mortalitet za djecu 1000 g je bio 3,0‰, a za djecu 500 g 4,2‰. Odgovarajući rani neonatalni mortalitet je bio 1,5‰ i 3,0‰. U porodu je umrlo 11 plodova, što je 0,24‰. Rani neonatalni mortalitet se smanjuje porastom težine novorođenčadi: za djecu 500–999 g je bio 483,0‰, za djecu 1000–1499 g 89,0‰, za 1500–1999 g 23,8‰, za 2000–2499 g 8,7‰ te za djecu 2500 g 0,6‰.U 15 od 35 rodilišta je standardni perinatalni mortalitet (djeca 1000 g) bio manji od 5,0‰, u 11 je bio 5,0–9,9‰, a u 4 bolnička i 5 izvanbolničkih rodilišta ni jedno dijete nije perinatalno umrlo. U trudnoći, porodu ili babinjama je umrlo 7 žena, pa je maternalna smrtnost u 2009. godini porasla od 6,9/100.000 živorođenih u 2008. godini na 15,6/100.000 živorođenih. Eklampsija je bilo 16 ili 0,36‰. Carskim rezom je dovršeno 8084 trudnoća, što je 18,2%, u blagom je porastu (za 0,45%) prema prethodnoj godini. Od svih SC bilo je 28,0% ponovljenih, a 69,95% od 3251 rodilje s ranijim carskim rezom je ponovno porođeno carskim rezom. Od 1614 jednoplodovih trudnoća u stavu zatkom njih 1232 (76.33%) je rođeno carskim rezom. Antenatalna skrb pokazuje stagnaciju prema prethodnoj 2008. godini: po trudnici je bilo prosječno 9,0±0,83 pregleda, 63,1% je pregledano 9 puta, od njih 28,1% više od 10 puta, ali još uvijek je 3,4% trudnica bilo bez pregleda ili s 1–2 pregleda, a 6,7% sa samo 3–5 pregleda. Prosječni broj pregleda ultrazvukom je bio 4,9±0,5, njih 70,0% je bilo pregledano 4 puta, od njih 46,1% 5 puta. U rodilištima III. razine s JINT, znakovito je niži fetalni mortalitet za djecu 500–1499 g. Organizacija perinatalne zaštite u Hrvatskoj u tri razine, s konačnim formiranjem jedinica intenzivne neonatalne terapije (JINT) i njege (JINNj), uz popunu tih jedinica opremom i uz odgovarajuću izobrazbu deficitarnih kadrova primarna su zadaća perinatalne zdravstvene skrbi., In the Republic of Croatia in the year 2009 died 7837 inhabitants more than were born, natality rate was 10,1 mortality rate 11.8, the population increase was negativ –1,8. The 45 056 infants with birthweight 500 grams (747 more than in previous 2008), 44853 infants with birthweight 1000 grams have been born. In the year 2009 the incidence of preterm infants was 5.44% and of those LBW-s 5.55%. Perinatal mortality for infants 1000 grams was 4.4‰ and for those 500 g 7.2‰. Fetal mortality for infants 1000 g was 3.0‰ and for those 500 g 4.2‰. The corresponding early neonatal mortality was 1.5‰ and 3.0‰. Eleven fetuses died intra partum i.e. 0.24‰. The early neonatal mortality decreases with increase of body weight: for infants 500–999 grams was 483.0‰, for those 1000–1499 g 89.0‰, for 1500–1999 g 23.8‰, for 2000–2499 g 8.7‰, and for infants 2500 grams 0.6‰. In 15 out of 35 maternity wards the perinatal mortality was

Rani neonatalni mortalitet (RNM) je sastavnica perinatalnog mortaliteta, zadnjih godina je manji od fetalnog mortaliteta. RNM je u 2009. godini u Hrvatskoj za djecu svih porodnih težina, uključujući onu s porodnom težinom ispod 500 grama bio 3,3‰, veći nego 2008. godine kad je bio 2,7‰. Neonatalni mortalitet (NM) je povećan od 3,1‰ u 2008. godini na 4,0‰. Mortalitet do otpusta iz bolnice je također povećan, od 3,5‰ u 2008. godini na 4,2‰. Najveći udjel povećanja mortaliteta može se pripisati povećanoj smrtnosti djece porodne težine, Early neonatal mortality (ENM) is one of components of perinatal mortality. In recent years ENM is smaller than the fetal one. ENM was in 2009 in Croatia for newborns of all birth-weights (BW), including those of BW

Here is presented a rare case of spontaneous closure of a fistula between the right coronary artery and the right ventricle in a female infant. At the age of 7 days continuous murmur intensity 3/6 was registered in the 3rd intercostal space along the right sternal border. Echocardiography indicated, with high probability, a coronary artery fistula. Definitive diagnosis was made at the age of 1 month with aortography. Radioscintiography and oximetric analysis revealed 47% of left-to-right shunting at level of fistula. Because of the size of the shunting a surgical correction had been planned, but in the mean-time the murmur had gone. At the age of 6 months control aortography showed spontaneous closure of the fistula, but with significant morphological changes in the right coronary artery (stenosis and aneurysms). The exact mechanism of spontaneous closure is unknown, as is the timing of therapeutic intervention. Control coronary angiography after 1 year showed the regression of pathological changes in the right coronary artery. Based on the literature and our own experiences we consider invasive therapy indicated in children with significant clinical symptoms. In cases were clinical symptoms are absent expectative state should be taken, because there is a chance of spontaneous closure of fistulas, even larger ones, as it is in our case.

Gljivama uzrokovana kasna novorođenačka sepsa u novorođenčadi rodne mase (RM), Late-onset fungal sepsis in very low birth weight (VLBW) newborns (BW

U 2008. godini je u Hrvatskoj umrlo 8398 stanovnika više, nego se rodilo, stopa nataliteta je bila 9,8‰, mortaliteta 11,8‰, negativni priraštaj iznosi –1,9.‰. Rođeno je 44033 djece ≥500 grama (2383 više nego 2007. godine), me|u njima 43833 djeteta ≥1000 grama. Učestalost nedonoščadi je 2008. godine bila 5,30%, a novorođenčadi niske porodne težine 5,23%. Perinatalni mortalitet za svu djecu ≥1000 g je bio 4,6‰, a za svu djecu ≥500 g 7,0‰. Fetalni mortalitet za djecu ≥1000 g je bio 3,5‰, a za djecu ≥500 g 4,5‰. Odgovarajući rani neonatalni mortalitet je bio 1,4‰ i 2,6‰. U porodu je umrlo samo 13 plodova, što je 0,23‰. Rani neonatalni mortalitet se smanjuje porastom težine novoro|enčadi: za djecu 500–999 g je bio 368,1‰, za djecu 1000–1499 g 60,6‰, za 1500–1999 g 17,6‰, za 2000–2499 g 3,1‰ te za djecu ≥2500 g 0,9‰. U 26 od 35 rodilišta je standardni perinatalni mortalitet (djeca >1000 g) bio manji od 5,0‰, u 9 je bio 5,0–9,9‰, a u 3 bolni~ka i 5 izvanbolničkih rodilišta ni jedno dijete nije perinatalno umrlo. U trudnoći, porodu ili babinjama su umrle 3 žene, pa je maternalna smrtnost u 2008. godini bila 6,9/100.000 živorođenih. Eklampsija je bilo 14 ili 0,329‰. Carskim rezom je dovršeno 7744 trudnoća, što je 17,75%, u blagom je porastu (za 0,96%) prema prethodnoj godini. Od svih SC bilo je 28,0% ponovljenih, a 67,5% od 3211 rodilje s ranijim carskim rezom je ponovno poro|eno carskim rezom. Antenatalna skrb pokazuje daljnji napredak prema prethodnoj 2007. godini: po trudnici je bilo prosječno 9,2 pregleda, 63,6% je pregledano ≥9 puta, od njih 28,9% više od 10 puta, ali još uvijek je 3,3% trudnica bilo bez pregleda ili s 1–2 pregleda, a 6,3% sa samo 3–5 pregleda. Prosječni broj pregleda ultrazvukom je bio 5,1, njih 76,5% je bilo pregledano ≥4 puta, od njih 49,2% ≥5 puta. U rodilištima III. razine, s JINT, znakovito je niži fetalni i rani neonatalni mortalitet za djecu 500–1499 g. Organizacija perinatalne zaštite u Hrvatskoj u tri razine, s konačnim formiranjem jedinica intenzivne neonatalne terapije (JINT) i njege (JINNj), uz popunu tih jedinica opremom i uz odgovarajuću izobrazbu deficitarnih kadrova primarna su zadaća perinatalne zdravstvene skrbi., In the Republic of Croatia in the year 2008 died 8398 inhabitants more than were born, natality rate was 9,8, mortality rate 11.8, the population increase was negativ, –1,9. The 44033 infants with birthweight ≥500 grams (2383 more than in previous 2007), 43833 infants with birthweight ≥1000 grams have been born. In the year 2008 the incidence of preterm infants was 5.30% and of those LBW-s 5.23%. Perinatal mortality for infants ≥1000 grams was 4.6‰ and for those ≥500 g 7.0‰. Fetal mortality for infants ≥1000 g was 3.5‰ and for those ≥500 g 4.5‰. The corresponding early neonatal mortality was 1.4‰ and 2.6‰. Thirteen fetuses died intra partum i.e. 0.23‰. The early neonatal mortality decreases with increase of body weight: for infants 500–999 grams was 368.1‰, for those 1000–1499 g 60.6‰, for 1500–1999 g 17.6‰, for 2000–2499 g 3.1‰, and for infants ≥2500 grams 0.9‰. In 26 out of 35 maternity wards the perinatal mortality was

Rani neonatalni mortalitet (RNM) je sastavnica perinatalnog mortaliteta, zadnjih godina je manji od fetalnog mortaliteta. RNM je u 2007. godini u Hrvatskoj bio 69% za skupinu novorođenčadi porodne težine (PT) 500–749 g, 30% za novorođenčad PT 750–999 g, dok je 2008. godine RNM za djecu PT ≤999 g bio 42%. Po 100-gramskim razredima RNM je bio za djecu PT, Early neonatal mortality (ENM) is one of components of perinatal mortality. In recent years ENM is smaller than fetal mortality. ENM was in 2008 in Croatia 69% for newborns of birth-weight (BW) 500–749 g; 30% for those 750–999 g, 16% for those 1000–1249 g, and 6% for newborns of BW 1250–1499 g. In newborns divided by 100-grams, ENM for infants BW

Growth factors from neural tissues have been described as potent mitogens for a wide variety of mesoderm- and ectoderm-derived cells in vitro. We used porcine brain extract for in vitro testing of proliferation properties on primary ovarian cells, uterine cells, and cardiomyocytes in culture as well as for BHK-21 [C-13] cell line. The addition of this extract accelerates proliferation in all examined cultures. It also lowers serum requirement and shortens the cultivation period for BHK-21 [C-13] cells. Fibroblast growth factors from brain of different species, but not porcine, are already characterized and their proliferative effect proved. Therefore, we purified, determined, and confirmed the presence of basic fibroblast growth factor in porcine brain extract by Western blot analysis and showed its biological activity on BHK-21 [C-13] cells.

We present a family form of balanced translocation t(7;14) found in a mother and her two sons. The mother had an aortic cardiac murmur, without hemo-dynamic repercussions and the children had almost identical clinical findings, significant supravalvular aortic stenosis, left ventricle intracavitary stenosis and multiple peripheral pulmonary stenosis but with no other clinical manifestations of Williams-Beuren syndrome, except, perhaps, a deep, metallic voice. The conventional chromosome analysis unexpectedly revealed a balanced translocation between chromosomes 7 and 14, the same translocation was found in the mother and both children. Subsequent fluorescent in situ hybridization analysis with WSCR probe showed that the break point was within the elastin gene region in the proband, in the mother and both children. The proband karyotype was interpreted, according to ISCN (2005) as 46,XY,t(7;14)(q11.23;p12).isht(7;14)(D7Z1+,ELNsp;D14Z1/D22Z1+,ELNsp+)mat, in other words, translocation had disrupted the elastin region and may have contributed to the developmental defects in Williams-Beuren syndrome. When going through the references on genetic examination of supravalvular aortic stenosis, Williams-Beuren syndrome and some other conditions that could not be placed in any of these two terminal categories because of the various phenotype characteristics, we found that such a result has not yet been published., Prikazujemo obiteljski oblik balansirane translokacije t(7;14) koji je nađen u majke i dva njezina sina. Majka je imala samo šum nad aortnim ušćem bez hemodinamskog značenja, djeca su imala gotovo istovjetan klinički nalaz; značajnu supravalvularnu aortnu stenozu tipa pješćanog sata, intrakavitarnu stenozu u lijevoj klijetki i multiple periferne pulmonalne stenoze. Nisu imali niti jedan drugi klinički znak Williams-Beurenova sindroma, osim vjerojatno dubokog metaličnog glasa. Konvencionalna kromosomska analiza neočekivano je pokazala da je riječ o balansiranoj translokaciji t(7;14), identična translokacija pronađena je u majke i brata. Fluorescentna in situ hibridizacija s WSCR probama pokazala je da je prijelomna točka uzdužno pocijepala elastinsku regiju u sve troje ispitanika. Kariotip ispitanika interpretiran je prema ISCN-u kao 46,XY,t(7;14)(q11.23;p12).ish t(7;14)(D7Z1+,ELNsp;D14Z1/D22Z1+,ELNsp+)mat. Drugim riječima, translokacija je pocijepala elastinsku regiju, što može biti razlogom nastanka razvojnih anomalija karakterističnih za Williams-Beurenov sindrom. Pregledom kroz literaturu našli smo da ovakav nalaz nije dosad objavljen u genetičkoj obradi supravalvularne aortne stenoze, odnosno Williams-Beurenova sindroma ili stanja koja se ne mogu razvrstati u ove dvije krajnje kategorije zbog šarolikosti fenotipskih karakteristika.

UENPS.85 Mortality of newborns to discharge from hospital in Croatia in 2006 Boris Filipovic-Grcic⁎, Hrvoje Kniewald, Ruza Grizelj, Urelija Rodin, Jasminka Stipanovic-Kastelic, Emilja Juretic, Milan Stanojevic, Vesna Benjak, Furlan Ivanka Antoncic, Igor Prpic, Kokeza Jadranka Blaskovic, Zora Zakanj, Jurica Vukovic, Dorotea Ninkovic, Dorotea Bartonicek NICU, Zagreb, Croatia Department of Public Health, Zagreb, Croatia NICU, Split, Croatia NICU, Rijeka, Croatia NICU, Osijek, Croatia Background and aim In recent years perinatal mortality in Croatia is reduced from about 10 to almost 5/1000. Early neonatalmortality (ENM) isoneof components of perinatal mortality. ENM is smaller than fetal mortality. Aim of this investigation is to analyze if lower counts of perinatal mortality in last years in Croatia are underestimated, and to find possible excess ofmortality of liveborn infants from perinatal causes later in the course of initial treatment. Materials and methods Perinatal report data are collected from all hospitals in the country, and analyzed the proportion of liveborns who died according to the age at death.

Objectives and study : Cystic cystitis is a separate form of urinary bladder inflammation, detected by cystoscopy in children with recurrent urinary infections. Cystoscopy is an invasive method, so the aim of this investigation was to determine the ultrasonographic characteristics of cystic cystitis and to assess the reliability of ultrasound in relation to cystoscopy in diagnosing cystic cystitis. Methods : The study included 115 girls with repeated urinary infections. Cystoscopy and ultrasonography was performed in all. According to the cystoscopic finding the subjects were divided into 4 groups. Lateral and posterior urinary bladder wall thickness was measured during ultrasonography. Volume of the liquid introduced into the bladder during cystoscopy and the ratio of this volume to the expected bladder capacity were determinated. Results : A statistically significant difference was found between all 4 groups, the method demonstrated a high degree of sensitivity (0.97) and specificity(0.91). Bladder wall thickness of 3.0 mm is presumed a borderline measure. In the group with normal cystoscopic finding the mean value of bladder wallthickness is 2.6 mm, in the group with less than 5 cysts it is 3.5 mm, in the group with 5 - 10 cysts it is 4.4 mm and in the group with more than 10 cysts the mean value is 4.8 mm. The probability that a positive test confirms the disease (LPR) is 10.95, and the ability for a negative test to confirm health (LNR) is 36, calculated for all groups. Percentile calculations were determined for wall thickness. Conclusions : Ultrasonography can replace endoscopy in diagnosis and follow-up of cystic cystitis in children, with at least 50% fullness of the urinary bladder as a prerequisite.

The study deals with the incidence and clinical significance of mitral valve prolapse in the population of outpatient pediatric cardiology patients in the time period from November 1999 to April 2004. The sample included 1187 children of both sexes, 688 of whom had a structural cardiac anomaly (57.9%), and the prolapse was diagnosed in 51 children (4.3%), largely female (f:m = 7.5:1). The average age at establishing diagnosis was 12.4 +/- 2.9 years (range 6-19 years). The children were followed 3.1 +/- 0.9 years (1-4 years of age). In 25 children (49%) associated mitral valve insufficiency was found, mostly of the 1st degree (80%). During follow up, neither the progression of the insufficiency nor any other complication was observed (arrhytmia, tromboembolism) in any of the children. Dolichostenomely was found in 10 children (19.6%), and both the development of insufficiency (p = 0.464, df = 2, chi2 = 1.54) and the difference in constitution (p = 0.766, df = 4, chi2 = 1.83) were irrelevant of sex. Typical subjective symptoms were observed in 37 children (72.5%), 22 of whom were treated with beta-blockers (propranolol) (43.1%). The average age of the patients treated with 3-blockers (13.7 +/- 2.5 yr) was statistically different from the average age of untreated patients (11.5 +/- 2.9 yr), hence the probability of the influence of neurohormonal factors on the development of subjective symptoms in advanced puberty (p = 0.006, t = -2.86). The most common clinical symptom is chest-pain (95% of the group with stronger symptoms). When treated, the symptoms disappear in 82% of the patients. Mitral valve prolapse is the entity of favourable clinical course. The prophylaxis of infective endocarditis should be performed in the group with mitral insufficiency, and the children with stronger symptoms should be treated with beta-blockers.

Rani neonatalni mortalitet (RNM) je sastavnica perinatalnog mortaliteta, i zadnjih godina je manji od fetalnog mortaliteta. RNM je u 2005. g. u Hrvatskoj bio 71% za skupinu novorođenčadi porodne težine (PT) 500–749 g, 32% za novorođenčad PT 750–999 g, 16% za novorođenčad PT 1000–1249 g, i 9% za novorođenčad PT 1250–1499 g. Mortalitet do otpusta iz bolnice (MOB) je za skupine novorođenčadi iste porodne težine bio 84%, potom 48%, zatim 20% i 10%. U skupinama novorođenčadi veće porodne težine RNM i MOB bili su još niži. RNM sve novorođenčadi >500 g bio je 3,4‰, neonatalni mortalitet (NM) je bio 4,1‰ i MOB je bio 4,4‰. RNM novorođenčadi PT >1000 g bio je 2,2‰, NM je bio 2,6‰ i MOB je bio 2,9‰. RNM je za svu novorođenčad PT >500 g činio prosječno 76,7% smrtnosti (145/189), dok je ostalih 44 djece (23,3%) umrlo nakon prvog tjedna života. To ukazuje da RNM nije podcijenjen na račun visokog kasnijeg moraliteta, i da pedijatrijska-neonatalna služba ne ostvaruje smanjenje RNM na račun kasnijeg povišenja ¬mortaliteta ili MOB. U 2005. godini je kao i u 2003. i 2004. tri četvrtine novorođenčadi PT 500–1499 g rođeno u rodilištima III. razine. Za vjerodostojnu analizu podataka ishoda novorođenčadi i djece planirana je izrada novih obrazaca perinatalnih zbivanja, uz prikupljanje podataka o postnatalnom transportu novorođenčadi i mjestu liječenja novorođenčeta. Potrebno je nastaviti prikupljati detaljne podatke o vitalnim događajima do otpusta iz bolnice. Ti podatci predstavljat će osnovu za planiranje potreba neonatološke službe, izradu smjernica za prenatalno i postnatalno usmjeravanje novorođenčadi i za davanje vjerodostojnijih prognoza roditeljima novorođenčadi najnižih porodnih težina., Early neonatal mortality (ENM) is one of components of perinatal mortality. In recent years ENM is smaller than fetal mortality. ENM was in 2004 in Croatia 71% for newborns of birth-weight (BW) 500–749 g; 32% for those 750–999 g, 16% for those 1000–1249 g, and 9% for newborns of BW 1250–1499 g. Mortality to discharge from hospital (MDH) for newborns in these birth-weight groups was 84%, 48%, 20% and 10%, respectively. In groups of newborns with larger BW over 1500 g ENM and MDH were even lower. ENM for all newborns BW >500 g was 3,4‰, neonatal mortality (NM) was 4,1‰, and MDH was 4,4‰, respectively. ENM for newborns of BW > 1000 g was 2,2‰, NM was 2,6‰, and MDH was 2,9‰, respectively. ENM made 76,7% mortality of all newborns (BW >500 g) (145/189), while the rest of 44 newborns (23,3%) died after the first week of life. Therefore, ENM was not underestimated due to possible higher late neonatal mortality, pediatric-neonatal services didn't reduce ENM on expenses of higher late neonatal mortality or MDH. In the year 2005, as in 2003 and 2004, three fourths of newborns of BW 500–1499 grams were born in maternities of 3rd level. Within the aims of the proper analysis of newborns’ outcome data, is creation of new certificates of vital events with details of postnatal transport and place of treatment of newborn. It is necessary to continue to follow survival or mortality of all newborns to discharge from the hospital. These data will give us benchmark for planning of neonatal resources, development of recommendations in perinatology-neonatology for prenatal and postnatal transfer, and for more exact prognoses of the smallest newborns in the process of decision making.

The purpose of this work was to show the importance of myocardial bioptate analysis using different methods in the diagram of diagnostic flow in primary cardiomyopathies in children. According to the guidelines of the Task Force on Cardiomyopathies of the WHO/ISFC, we identified 121 children (50 f and 71 m) as having cardiomyopathy, giving an average occurrence for all cardiomyopathies of 38.81 for each 10,000 pts examined in our outpatient clinics for paediatric cardiology. The dilated cardiomyopathy (DCM) was identified in 52 pts (42.9%), hypertrophic cardiomyopathy (HCM) in 43 pts (35.5%) and restrictive cardiomyopathy (RCM) in 6 pts (4.8%). We placed 11 pts (9.0%) in the group of specific cardiomyopathies. In nine pts (7.4%), it was impossible to classify the cardiomyopathy. Most of those with DCM had been diagnosed prior to the age of 3 years (RR 1.9, 95% CI 1.4-2.47). There were no statistically significant differences in the incidences of DCM as compared to HCM (Z 0.923, p0.1779), but we encountered a significantly lower occurrence of RCM (Z 6.044, p0.001). The biopsy of endocardium and myocardium was done to confirm the etiology of primary cardiomyopathy in 22 pts, 12 m and 10 f, age 1 to 17 (average age 9.5y). The bioptates were analysed by light microscope (Dallas criteria) in all pts, 13 bioptates by direct immunofluorescence, 8 by immunohystochemical method (two hystochemically by the method of coloring with Kongo red, one by the microscopy in polarised light), 7 by electron microscope, and 5 by PCR method where DNA and RNA of cardiotrophic viruses was used. Out of 10 pts with DCM, in 4 myonecrosis as a consequence of acute myocarditis and in 6 signs of late inflammatory processes, as a consequence of chronic immunologic myocarditis, were found. In 4 of them rebiopsy proved complete healing. In 5 pts with HCM the diagnosis was confirmed hystologically. One bioptate was analysed by electron microscope to rule out mitochondriopathy. Out of 4 pts with RCM due to inflammation, in 3 pathohistological findings proved diagnosis and in one showed primary amyloidosis. In one patient pathohystological finding showed fibroelastosis. In one patient heart tumor (fibroma) has been found.

Rani neonatalni mortalitet (RNM) je sastavnica perinatalnog mortaliteta, zadnjih godina je manji od fetalnog¬ mortaliteta. RNM je za 2003.g. u Hrvatskoj bio 76% za skupinu novorođenčadi porodne težine (PT) 500–749 g, 53% za novorođenčad PT 750–999 g, 19% za novorođenčad PT 1000–1249 g, i 4% za novorođenčad PT 1250–1499 g. Neonatalni mortalitet (NM) je za skupine novorođenčadi iste porodne težine bio 81%, potom 70%, zatim 25% i 5%. Kasni neonatalni mortalitet (KNM) u novorođenčadi PT 500–999 g iznosi najviše 12,3%, dok je u skupini novorođenčadi PT 1000–1499 g najviše 9,1%. To pokazuje da RNM nije podcijenjen na račun visokog KNM, i da pedijatrijska – neonatalna služba ne ostvaruje smanjenje RNM na račun kasnijeg povišenja KNM. U neposrednoj budućnosti treba u cijeloj državi ostvariti praćenje preživljavanja sve novorođenčadi do otpusta iz bolnice. Ti podatci predstavljat će osnovu za planiranje potreba neonatološke službe, izradu smjernica za prenatalno i postnatalno usmjeravanje novorođenčadi i za davanje vjerodostojnijih¬ prognoza roditeljima novorođenčadi najnižih porodnih težina., Early neonatal mortality (ENM) is one of components of perinatal mortality. In recent years ENM is smaller than fetal mortality. ENM was in 2003 in Croatia 76% for newborns of birth-weight (BW) 500–749 g; 53% for those 750–999 g, 19% for those 1000–1249 g, and 4 % for newborns of BW 1250–1499 g. Neonatal mortality for newborns in these birth-weight groups was 81%, 70%, 25% and 5% respectively. Late neonatal mortality (LNM) is maximal 12.3% for newborns of BW 500–999 g, and 9.1% for newborns of BW 1000–1499 g. ENM was not underestimated instead of possible higher LNM, pediatric-neonatal services didn’t reduce ENM on expenses of LNM. In close future is necessary to follow survival or mortality of all newborns up to discharge from hospital. These data will give us benchmark for planning of neonatal resources, development of recommendations in perinatology-neonatology for prenatal and postnatal transfer, and for more exact prognoses to parents of the smallest newborns.

Juvenile xanthogranuloma (JXG) presents a normolipemic non-Langerhans cells histiocytosis. JXG usually presents with cutaneous lesions. Visceral involvement is rare but may affect various organs. Deep JXG differs histologically from the cutaneous form by its tendency to consist solely of homogeneous proliferation of histiocytes without any xanthomatous or Touton giant cells. Awareness of the possibility of this atypical presentation of JXG helps in making the correct histologic diagnosis, which is supported by proving adequate immunomarkers on histiocytes (mainly PG-M1, an antibody against the CD68 antigen). JXG may present with intramuscular lesions only; however, rarely JXG has been reported to affect the heart but not without the typical cutaneous manifestations. We present an unusual case of deep JXG without systemic disease or metabolic abnormalities. To our knowledge, this is a first reported case of intracavitar JXG without skin lesions.

) for intradermal skin test. Methods: We performed a prospective, randomized study in which healthy adult volunteers were divided into the two analgesic pretreatment groups (ice cube group and 4% lidocaine cream group). We chose the same intervention site of anterior aspect of left forearm in both groups. As a pretreatment, an ice cube in the ice cube group and 5 mg of 4% lidocaine cream in the 4% lidocaine cream group was applied, respectively. Intradermal skin test was performed 1 minute after application of an ice cube, and 40 minutes after application of 4% lidocaine cream, considering the respective time to have an enough analgesic effect. 5 minutes after intradermal skin test, pain was assessed using a 100 mm visual analogue scale (VAS). VAS score of each group was compared using Mann-Whitney U test. Results: A total of 35 volunteers were participated in this study (17: ice cube group, 18: 4% lidocaine cream group). There were no differences in demographic characteristics. The Median VAS score was 20 (0, 35) in the ice cube group and 70 (50, 80) in the 4% lidocaine cream group (p

Depending on the concentration, catecholamines activate various intracellular signaling pathways and can induce apoptosis in cardiac myocytes. Although 5, 5’ , 6, 6’ -tetrachloro-1, 1’ , 3, 3’ -tetraethylbenzimidazolocarbocyanine iodide (JC-1) has been previously used to study mitochondria in intact cardiomyocytes, there have been no reports on the detection of apoptosis in neonatal cardiomyocytes in combination with flow cytometry and confocal microscopy. In our study, neonatal rat cardiomyocytes were exposed to norepinephrine (NE) and isoproterenol (ISO) in concentrations of 1 microM and 10 microM for 48 hours. NE concentrations of 1 microM and 10 microM decreased the number of viable cardiomyocytes by 18% (*p